Polycythemia vera ndi matenda omwe mafupa ambiri amapanga maselo ofiira ambiri, maselo oyera, ndi mapulateletti, omwe angapangitse ngozi yowonjezera magazi.
Kukhala ndi maselo ambiri ofiira a m'magazi ndi chizindikiro chodziwika bwino cha polycythemia vera. Kusintha kwa jini kwa JAK2, mapuloteni omwe amagwiritsidwa ntchito pozindikiritsa mkati mwa selo, wapezeka mwa anthu ambiri omwe ali ndi vutoli.
Ndani Amalandira Polycythemia Vera?
Polycythemia vera ikhoza kuchitika pa msinkhu uliwonse koma nthawi zambiri imachitika m'moyo. Ngati muyang'ana zaka za anthu onse omwe ali ndi vutoli, chiwerengero cha pakati pa zaka zakubadwa chidzakhala zaka 60, ndipo sichikuwoneka mwa anthu omwe ali ndi zaka zoposa 40. Chiwerengero cha polycythemia vera ndi chapamwamba kwambiri kuposa amuna ndipo ndipamwamba kwambiri kwa amuna azaka 70 mpaka 79.
Anthu Ambiri Amakhudzidwa Bwanji?
Zili zovuta kunena motsimikiza, popeza munthu akhoza kukhala ndi vutoli kwa nthawi yaitali osadziŵa, koma chiwerengero ndi pafupifupi mmodzi kapena awiri mwa 100,000.
Malinga ndi Incyte Corporation, kampani ya Wilmington, Delaware, yochokera ku biopharmaceutical katswiri wodziwika bwino pa zamagetsi, pali anthu pafupifupi 25,000 ku United States omwe amakhala ndi polycythemia vera ndipo amaonedwa kuti sakulamuliridwa chifukwa amayamba kutsutsa kapena kusagwirizana ndi mankhwala ochizira, hydroxyurea .
Incyte ikugwirizanitsa ndi pafupifupi 200 magulu a zachipatala komanso maphunziro azachipatala kuti azichita maphunziro ku US otchedwa REVEAL, omwe adayamba kulembetsa odwala, ndipo akuyembekezeredwa kuti akhale odwala 2,000 omwe akuyang'aniridwa ndi dokotala kuti athetse matendawa kwa nthawi kwa zaka zitatu.
Kodi Ndi Khansa Kapena Matenda?
Polycythemia vera ali ndi zikhalidwe zina zofanana ndi khansara pomwe zimaphatikizapo kugawidwa kosasamalidwa kwa maselo achilendo ndipo sizitsitsika. Kudziwa izi, kuphunzira kuti inu kapena wokondedwa wanu muli ndi matendawa kungakhale kovuta. Komabe, dziwani kuti vutoli likhoza kuyendetsedwa bwino kwa nthawi yaitali.
Nyuzipepala ya National Cancer Institute imatanthauzira polycythemia vera motere: "Matenda omwe ali ndi maselo ofiira ambiri m'magazi ndi mwazi, zomwe zimayambitsa magazi. Chiwerengero cha maselo oyera ndi maselo angaperekedwe. Maselo ena owonjezera amatha kusonkhanitsa mumphuno ndikupangitsa kuti awoneke. Zingayambitsenso mavuto a magazi ndipo zimapanga mawotchi m'magazi. "
Malinga ndi The Leukemia & Lymphoma Society, anthu omwe ali ndi polycythemia vera ali pangozi yochepa kusiyana ndi anthu ambiri omwe akudwala matenda a khansa chifukwa cha matendawa komanso / kapena mankhwala enaake omwe alipo. Ngakhale kuti matendawa ndi osalephereka, kumbukirani kuti nthawi zambiri amatha kuyendetsedwa bwino kwa nthawi yayitali, ndipo safupikitsa nthawi ya moyo. Kuonjezerapo, mavuto amatha kuchiritsidwa ndi kutetezedwa ndi kuyang'anira zachipatala.
Kodi Zimapita Patsogolo?
Inde, koma mfundo zokhudzana ndi chiopsezo cha kupita patsogolo zidakali kufufuzidwa. Ngakhale kuti anthu sangakhale ndi zizindikiro kwa zaka zambiri, polycythemia vera ikhoza kutsogolera zizindikiro ndi zizindikilo zingapo, kuphatikizapo kutopa, kutentha, kutuluka usiku, kupweteka m'mafupa, kutentha thupi ndi kutaya thupi. Pafupifupi 30 peresenti mpaka 40 peresenti ya anthu omwe ali ndi polycythemia vera ali ndi nthonje yofutukuka. Kwa anthu ena, izo zingayambitse zizindikiro zofooketsa ndi mavuto a mtima. Matenda a matendawa adakali wofufuzidwa mwakhama.
Kodi Zimapezeka Bwanji?
Kafukufuku wotchedwa hematocrit concentration amagwiritsidwa ntchito pozindikira polycythemia vera ndikuyesa yankho la munthu kuchipatala.
Hematocrit ndi kuchuluka kwa maselo ofiira ofiira m'magazi ambiri, ndipo nthawi zambiri amaperekedwa monga peresenti kapena kuwonjezeka kwa magazi m'magazi.
Anthu omwe ali ndi thanzi labwino, amatha kukhala pakati pa 36 ndi 46 peresenti mwa amayi ndi 42 mpaka 52 peresenti mwa amuna. Zina zowonjezereka zomwe zingakunkhidwe kuchokera ku kuyesedwa kwa magazi zimathandizanso pa matendawa, kuphatikizapo kukhalapo kwa kusintha-JAK2 kusintha-m'magazi a maselo. Ngakhale kuti sikofunika kuti apange matendawa, anthu ena angakhalenso ndi kufufuza kwa mongofu monga gawo la kuponyedwa kwawo ndi kuyesa.
Kodi Zimakhudzidwa Bwanji?
Malingana ndi The Leukemia & Lymphoma Society , phlebotomy, kapena kuchotsedwa kwa magazi kuchokera mu mitsempha, ndi njira yachizolowezi yoyamba ya chithandizo kwa odwala ambiri. Izi zikhoza kuchepetsa ndondomeko ya hematocrit, yomwe imabweretsa kusintha kwa zizindikiro zina monga mutu, kumveka m'makutu, ndi chizungulire.
Mankhwalawa angakhale ndi othandizira omwe amatha kuchepetsa maselo ofiira kapena mapuloteni omwe amatchedwa odwala matendawa. Ambiri omwe amagwiritsa ntchito myelosuppressive agent kwa polycythemia vera ndi hydroxyurea, operekedwa ndi pakamwa. Zotsatira zofala zambiri zimaphatikizapo chifuwa kapena kutentha, malungo kapena chifuwa, kupweteka kumbuyo kapena kupweteka kwa pambali, komanso kupweteka kovuta.
About Jakafi kwa Polycythemia Vera
Ruxolitinib (Jakafi) ndi mankhwala omwe amagwiritsidwa ntchito pochiritsa anthu omwe ali ndi polycythemia vera amene atenga kale hydroxyurea ndipo sizinagwire ntchito mokwanira kapena sakanatha kupirira. Jakafi amagwiritsidwanso ntchito pochitira mtundu wina wa myelofibrosis -kusowa kwa mafupa.
Malinga ndi a FDA, Jakafi amagwira ntchito poletsa mazira ochedwa Janus Associated Kinase (JAK) 1 ndi 2 omwe akukhudzidwa kuti athetse magazi ndi maumunthu. Chivomerezo cha mankhwala pochiritsa polycythemia vera chidzathandizira kuchepetsa zochitika za nthenda yotukulidwa-splenomegaly-ndi kufunikira kwa phlebotomy, njira yochotsera magazi owonjezera kuchokera mu thupi.
Zotsatira zambiri zomwe zimagwirizanitsidwa ndi kugwiritsidwa ntchito kwa Jakafi mwa ophunzira ndi polycythemia vera m'mayesero oyenerera opaleshoni ndi ochepa kwambiri a maselo ofiira magazi (anemia) ndi chiwerengero chochepa cha magazi (thrombocytopenia). Zotsatira zowonjezereka zosagwirizana ndi magazi ndizozunguza, kudzimbidwa, ndi kumangirira.